Neuromyelitis optica

The neuromyelitis (NMO abbreviated, also Devic's syndrome) optica is an autoimmune inflammatory disease of the central nervous system with degradation of the insulating nerve sheaths ( demyelination ), in most cases within a few months ( usually < 2 years) consecutively or simultaneously both an inflammation of at least one optic nerve ( optic neuritis ) and inflammation of the spinal cord ( myelitis ) occurs. Very often there are antibodies against the water channel aquaporin- fourth What pathophysiological role of these antibodies is still the subject of current research.

Epidemiology

The disease is rare ( approximately 1% of demyelinating diseases). It is controversial whether NMO a special form of multiple sclerosis ( MS), or a standalone disorder.

Signs of disease

• Impaired vision and even blindness ( amaurosis ) of an eye or both eyes within hours to days
• Spinal cord injury with partial ascending symptoms (eg paresthesia, weakness / paralysis of the extremities, bladder dysfunction, and others)

Diagnosis

Diagnosis is initially clinical, ie to make it based on health survey (anamnesis) and neurological examinations, by looking for symptoms of optic neuritis and spinal cord involvement and symptoms that are due to lesions in the brain, are excluded. To confirm the diagnosis, the determination of aquaporin - 4 antibodies and magnetic resonance imaging of the skull and spine are necessary and for the differential diagnosis, a lumbar puncture, evoked potentials and possibly electromyography / neurography.

A safe delineation of multiple sclerosis is not always possible at the beginning of the disease. If a patient develops over the course additionally symptoms that can infer an involvement of the brain outside of the optic nerves, so this represents the diagnosis in question. Another syndrome, retrobulbar neuritis, can be expressed in terms of the vision as well, but remains by definition without spinal cord involvement.

Course and treatment

The disease often runs with only a single disease flare ( monophasic ), but can also occur in several stages or chronic progressive. Histologically, demyelination ( similar to those in multiple sclerosis ), often regress good. However, one also damage coming through tissue necrosis ( necrotizing injury ) prior to permanent damage.

The treatment is carried out with cortisone or in the absence of shear response, which is more common than in multiple sclerosis, with plasmapheresis. The long-term treatment, in contrast to multiple sclerosis, where immune modulators are used in the first place, especially in immunosuppression, such as azathioprine. In a first study, also the use of the antibody rituximab seemed quite promising.

History

The disease was described in the first half of the 19th century for the first time. Later, Eugène Devic and his student Fernand Gault tried to systematize the knowledge, which is why the disease also Devic's syndrome is named.

Study Group

The NMO Study Group ( NEMOS ) networked clinical and scientific activities to NMO for doctors and patients to obtain important information about the epidemiology and clinical course.